Is single-daily low-dose cyclosporine therapy really effective in children with idiopathic frequent-relapsing nephrotic syndrome?
Is single-daily low-dose cyclosporine therapy really effective in children with idiopathic frequent-relapsing nephrotic syndrome?
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每日单次小剂量环孢素治疗对特发性频繁复发肾病综合征患儿真的有效吗？
DOI：
10.5414/cnp69084
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发表时间：
2008
影响因子：
1.1
通讯作者：
K. Kaneko
中科院分区：
文献类型：
--
作者：
S. Fujinaga;Y. Ohtomo;T. Someya;T. Shimizu;Y. Yamashiro;K. Kaneko
背景
最近对肾移植患者的一项研究表明，单剂量环孢素（CsA）增加了减少剂量和副作用的优势，同时保持移植功能。然而，这种方案在儿童特发性频繁复发性肾病综合征（NS）的疗效仍存在争议。
方法
这项前瞻性研究招募了20名类固醇依赖性NS或CsA依赖性NS儿童（18名患有微小病变疾病（MCD），2名患有局灶性节段性肾小球硬化症（FSGS））。CsA开始剂量为1.5 - 2 mg/kg，早餐前每日单次给药，剂量调整至给药后2小时CsA水平（C2）为600 - 800 ng/ml。
结果
18例MCD患者中有9例接受CsA每日一次治疗，中位时间为13个月（范围7 - 21）导致平均最小泼尼松龙（PSL）剂量从1.1 A +/-0.55 mg/kg减少至0.04 A +/-0.09 mg/kg，隔日一次中位复发率为1.3（1.1~2.5）~0（0~0.2）次/6个月（p <0.01）。其中7例患者（6例激素依赖型NS中4例，14例CsA依赖型NS中3例）在此治疗过程中可停用PSL而无NS复发。然而，20例患者中有11例被认为治疗失败：1例为类固醇依赖性NS，10例为CsA依赖性NS。在2名患有FSGS的患者中，这种方法没有显示出有益的效果。在18例MCD患者中，CsA每日一次治疗的无复发率在平均C2水平大于700 ng/ml的患者中显著较高（p <0.05）。
结论
我们的经验表明，维持C2水平高于700 ng/ml的每日一次低剂量CsA治疗可能对患有类固醇依赖性NS或MCD的儿童有效，并且不会复发。相比之下，这种治疗方案在CsA依赖性NS儿童中的有效性似乎有限。
BACKGROUND
A recent study on renal transplant patients has shown that a single dose of cyclosporine (CsA) has added the advantage of decreasing dosages and adverse effects, while maintaining graft function. However, the efficacy of this regimen in children with idiopathic frequent-relapsing nephrotic syndrome (NS) remains controversial.
METHODS
20 children with steroid-dependent NS or CsA-dependent NS (18 with minimal change disease, MCD and 2 with focal segmental glomerulosclerosis, FSGS) were enrolled in this prospective study. CsA was commenced at 1.5 â 2 mg/kg, given as a single daily dose before breakfast, and the dose was adjusted to reach 2 hours post-dose CsA levels (C2) of 600 - 800 ng/ml.
RESULTS
In 9 out of 18 patients with MCD, treatment with single-daily CsA for a median of 13 months (range 7 - 21) resulted in a reduction of mean minimum prednisolone (PSL) dose from 1.1 A+/- 0.55 to 0.04 A+/- 0.09 mg/kg on alternate days (p < 0.01), and the median relapse rate from 1.3 (1.1 - 2.5) to 0 (0 - 0.2) episodes/6 months (p < 0.01). Of them, PSL could be weaned off in 7 patients (4 of 6 with steroid-dependent NS, only 3 of 14 with CsA-dependent NS) without relapse of NS while on this therapy. However, 11 out of 20 were considered to have treatment failure: 1 with steroid-dependent NS and 10 with CsA-dependent NS. In 2 patients having FSGS, this method showed no beneficial effects. In 18 patients with MCD, relapse free ratio on single-daily CsA therapy was significantly higher in patients whose average C2 levels were greater than 700 ng/ml (p < 0.05).
CONCLUSIONS
Our experience demonstrates that single-daily low-dose CsA therapy maintaining C2 levels greater than 700 ng/ml may be effective in children with steroid-dependent NS or MCD, with no relapse. In contrast, the usefulness of this regimen in children with CsA-dependent NS appears to be limited.