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成人原发性局灶节段性肾小球硬化:治疗反应与预后

[Primary focal segmental glomerulosclerosis in adults: response to treatment and outcome].

作者信息

Niang A, Hachim K, Zahiri K, Fatihi E, Benghanem M, Ramdani B, Sqalli S, Zaid D

出版信息

Dakar Med. 2004;49(1):13-6.

Abstract

Response to therapy of primary focal segmental glomerulosclerosis (FSGS) in adults is poor, most studies demonstrated no response at all. We report our experience from a retrospective study about 22 adults patients. All patients presented proteinuria at admission with a nephrotic syndrome in 86 percent of cases. A high blood pressure was noted in 25 percent. A response to corticosteroid was obtained in 8 patients (36%) with complete remission in 5 cases (23%). Three patients were corticosteroid-dependant, two out of them went in remission under cyclophosphamide in one case and under chlorambucil in the other case. Fourteen patients did not respond to corticosteroids. Eight received cyclophosphamide with partial remission in 4 cases and progression to chronic renal failure (CRF) in 4 cases. The six non-responders did not receive immunosuppressive drugs because of severe CRF in 4 cases. The mean duration of follow-up was 39 months (20-55). The response to corticosteroid of primary FSGS in adults remain low (30%). The use of cyclophosphamide has improved the global remission to 50 percent.

摘要

成人原发性局灶节段性肾小球硬化(FSGS)对治疗的反应较差,大多数研究表明根本没有反应。我们报告了一项针对22例成年患者的回顾性研究的经验。所有患者入院时均有蛋白尿,86%的病例为肾病综合征。25%的患者有高血压。8例患者(36%)对皮质类固醇有反应,5例(23%)完全缓解。3例患者依赖皮质类固醇,其中2例分别在环磷酰胺或苯丁酸氮芥治疗下缓解。14例患者对皮质类固醇无反应。8例接受环磷酰胺治疗,4例部分缓解,4例进展为慢性肾衰竭(CRF)。6例无反应者因4例严重CRF未接受免疫抑制药物治疗。平均随访时间为39个月(20 - 55个月)。成人原发性FSGS对皮质类固醇的反应仍然较低(30%)。环磷酰胺的使用使总体缓解率提高到了50%。

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