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环孢素对儿童特发性肾病综合征的治疗是否具有真正的优势?一项长期疗效和安全性研究。

Does cyclosporine achieve a real advantage for treatment of idiopathic nephrotic syndrome in children? A long-term efficacy and safety study.

作者信息

Sheashaa Hussein, Mahmoud Ihab, El-Basuony Fathy, El-Husseini Amr, Hassan Nabil, El-Baz Mahmoud, Ahmed Nagy Sayed, Sobh Mohamed

机构信息

Nephrology unit, Urology & Nephrology Center, Mansoura, Egypt.

出版信息

Int Urol Nephrol. 2007;39(3):923-8. doi: 10.1007/s11255-007-9194-x. Epub 2007 Apr 20.

DOI:10.1007/s11255-007-9194-x
PMID:17447154
Abstract

BACKGROUND

Cyclosporine (CsA) was found to be efficient in decreasing proteinuria in both steroid-dependent and steroid-resistant nephrotic patients. We aimed to explore the potential long-term benefits and hazards of CsA and their predictors among a large group of nephrotic patients.

METHODS

In this retrospective analysis, we included 197 pediatric patients with idiopathic nephrotic syndrome (INS) of whom 103 were steroid dependent and 94 steroid resistant.

RESULTS

CsA induced complete remission in 132 (67%) and partial response in 13 (6.6%). Cyclosporine was received for a period of 22.16 +/- 12.21 months. Univariate analysis showed that the response to CsA was significantly better in steroid-dependent children, in minimal change disease (MCD), diffuse mesangial proliferative glomerulonephritis (DMP) and focal segmental glomerulosclerosis (FSGS) than in other pathological lesions and in those who had lower quantities of pretreatment proteinuria. Only the prior response to steroids and concomitant use of ketoconazole with CsA were valid predictors for better response to CsA with multivariate analysis. Discontinuation of the drug in 40 patients resulted in relapse in 26 patients while the remaining 14 patients maintained remission. Renal dysfunction developed in 18 patients of whom 12 recovered completely on drug discontinuation. Thirty-seven patients developed hypertension. Multivariate analysis showed that all side-effects were significantly more prevalent in CsA-resistant patients.

CONCLUSION

CsA is effective and well tolerated in the long-term treatment of INS in children, however two thirds of cases showed relapse after CsA discontinuation.

摘要

背景

环孢素(CsA)被发现对降低激素依赖型和激素抵抗型肾病患者的蛋白尿有效。我们旨在探讨一大群肾病患者中环孢素潜在的长期益处和危害及其预测因素。

方法

在这项回顾性分析中,我们纳入了197例特发性肾病综合征(INS)的儿科患者,其中103例为激素依赖型,94例为激素抵抗型。

结果

环孢素使132例(67%)完全缓解,13例(6.6%)部分缓解。环孢素的治疗时间为22.16±12.21个月。单因素分析显示,激素依赖型儿童、微小病变肾病(MCD)、弥漫性系膜增生性肾小球肾炎(DMP)和局灶节段性肾小球硬化(FSGS)患者对环孢素的反应明显优于其他病理病变患者以及那些治疗前蛋白尿水平较低的患者。多因素分析显示,只有既往对激素的反应以及酮康唑与环孢素联合使用是对环孢素反应更好的有效预测因素。40例患者停药后,26例复发,其余14例维持缓解。18例患者出现肾功能不全,其中12例停药后完全恢复。37例患者出现高血压。多因素分析显示,所有副作用在环孢素抵抗患者中更为普遍。

结论

环孢素在儿童INS的长期治疗中有效且耐受性良好,然而三分之二的病例在停用环孢素后复发。

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Long-term ciclosporine A treatment in adults with minimal change nephrotic syndrome or focal segmental glomerulosclerosis.环孢素A对成人微小病变型肾病综合征或局灶节段性肾小球硬化症的长期治疗。
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