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类固醇、环孢素和环磷酰胺在激素抵抗型特发性肾病综合征中的疗效

Efficacy of steroids, cyclosporin and cyclophos-phamide in steroid resistant idiopathic nephrotic syndrome.

作者信息

Hafeez Farkhanda, Ahmad Tahir Masood, Anwar Sumair

机构信息

Department of Paediatric Nephrology, The Children's Hospital and Institute of Child Health, Lahore.

出版信息

J Coll Physicians Surg Pak. 2005 Jun;15(6):329-32.

Abstract

OBJECTIVE

To determine the efficacy of three different treatment protocols in steroid resistant idiopathic nephrotic syndrome, (SRINS).

DESIGN

Interventional study.

PLACE AND DURATION OF STUDY

Department of Nephrology at The Children's Hospital, Lahore, over a period of 3 years from January 2000 to December 2002.

PATIENTS AND METHODS

Nephrotic children who did not respond to four weeks of steroid therapy (60mg/M2) followed by three pulses of methyl prednisolone (1Gm/1.73M2) over a period of one week were labeled as steroid resistant. Those with histopathological lesions of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and Mes.PGN were divided into 3 groups. Group-I was treated with cyclophosphamide (CPM) and oral steroids, group-II with cyclosporine and oral steroids and group-III with pulse methyl prednisolone (MPP) and oral steroid + CPM. The response to treatment and course of disease were observed in each group.

RESULTS

Twenty patients with mean age of 4.4 years were enrolled. On the whole 10 (50%) had complete remission. In group-I, 5 (50%), in group-II, 3 (75%) and in group-III, 2 (33.3%) had complete remission. Depending upon histological lesion 100% (n=2) with MCD, 50% (n=6) with Mes.PGN and 25% (n=1) with FSGS achieved complete remission. Cyclosporine and CPM induced remission in 100% of patients with MCD, while in Mes.PGN response rate in group-I, II, and III was 100% (n=1), 50%(n=1), and 44.4%(n=4) respectively. In patients with FSGS, MPP was the only drug used with limited response of 25% (n=1).

CONCLUSION

Cyclosporine proved to be a better option for MCD and Mes.PGN, while MPP showed limited response in patients with FSGS.

摘要

目的

确定三种不同治疗方案对激素抵抗型特发性肾病综合征(SRINS)的疗效。

设计

干预性研究。

研究地点及时间

拉合尔儿童医院肾病科,从2000年1月至2002年12月,为期3年。

患者及方法

对接受为期四周的激素治疗(60mg/M2)且随后在一周内接受三次甲泼尼龙冲击治疗(1Gm/1.73M2)后无反应的肾病患儿标记为激素抵抗型。将具有微小病变病(MCD)、局灶节段性肾小球硬化(FSGS)和系膜增生性肾小球肾炎(Mes.PGN)组织病理学病变的患儿分为3组。第一组用环磷酰胺(CPM)和口服激素治疗,第二组用环孢素和口服激素治疗,第三组用甲泼尼龙冲击治疗(MPP)加口服激素 + CPM。观察每组的治疗反应和病程。

结果

共纳入20例平均年龄4.4岁的患者。总体上10例(50%)完全缓解。第一组5例(50%),第二组3例(75%),第三组2例(33.3%)完全缓解。根据组织学病变,MCD患者100%(n = 2)、Mes.PGN患者50%(n = 6)、FSGS患者25%(n = 1)实现完全缓解。环孢素和CPM使100%的MCD患者缓解,而在Mes.PGN中,第一组、第二组和第三组的缓解率分别为100%(n = 1)、50%(n = 1)和44.4%(n = 4)。在FSGS患者中,MPP是唯一使用的药物,缓解率有限,为25%(n = 1)。

结论

环孢素被证明对MCD和Mes.PGN是更好的选择,而MPP在FSGS患者中显示出有限的反应。

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