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脉冲环磷酰胺治疗激素抵抗型局灶节段性肾小球硬化症。

Pulse cyclophosphamide for steroid-resistant focal segmental glomerulosclerosis.

作者信息

Rennert W P, Kala U K, Jacobs D, Goetsch S, Verhaart S

机构信息

Department of Pediatrics, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Johannesburg, South Africa.

出版信息

Pediatr Nephrol. 1999 Feb;13(2):113-6. doi: 10.1007/s004670050574.

Abstract

We report the response of ten patients (6 male, 4 female) with steroid-resistant focal segmental glomerulosclerosis (FSGS) to treatment with intravenous pulse cyclophosphamide (IVCP) together with oral prednisone. All patients had been treated with 60 mg/m2 oral prednisone daily for 2 months upon initial presentation. IVCP was given monthly at a dose of 500 mg/m2 over 6 months. Oral prednisone was given concurrently at 60 mg/m2 daily for 2 months and then on alternate days for 4 months, followed by 30 mg/m2 on alternate days for 6 months. Prednisone was then tapered monthly by 10 mg and finally discontinued. Five patients failed to respond to steroids from the onset and were considered as primary steroid resistant. Two of these patients achieved sustained remission after IVCP, one patient showed a partial response, with loss of edema, normalization of serum albumin, and persistent proteinuria, while two patients showed no response to IVCP. The other five patients had achieved remission after 2 months of daily prednisone at 60 mg/m2 upon initial presentation, but had suffered from more than three relapses per year and had eventually become steroid resistant. They were considered secondary steroid resistant. All five patients achieved sustained remission after IVCP. None of our patients suffered from adverse effects of IVCP. We suggest IVCP as an adjunctive therapy for steroid-resistant FSGS, particularly for patients with secondary steroid resistance.

摘要

我们报告了10例(6例男性,4例女性)激素抵抗型局灶节段性肾小球硬化(FSGS)患者接受静脉脉冲环磷酰胺(IVCP)联合口服泼尼松治疗的反应。所有患者初诊时均接受每日60mg/m²口服泼尼松治疗2个月。IVCP每月给药一次,剂量为500mg/m²,共6个月。同时口服泼尼松,每日60mg/m²,持续2个月,然后隔日服用4个月,随后隔日30mg/m²,持续6个月。之后泼尼松每月减量10mg,最终停药。5例患者从一开始就对激素无反应,被视为原发性激素抵抗。其中2例患者在接受IVCP治疗后实现持续缓解,1例患者有部分反应,水肿消退,血清白蛋白恢复正常,但仍有持续性蛋白尿,而2例患者对IVCP无反应。另外5例患者初诊时接受每日60mg/m²泼尼松治疗2个月后曾缓解,但每年复发超过3次,最终成为激素抵抗型。他们被视为继发性激素抵抗。所有5例患者在接受IVCP治疗后均实现持续缓解。我们的患者均未出现IVCP的不良反应。我们建议将IVCP作为激素抵抗型FSGS的辅助治疗方法,尤其是对于继发性激素抵抗的患者。

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