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脉冲式甲泼尼龙治疗特发性激素抵抗型肾病综合征

Pulse methylprednisolone treatment of idiopathic steroid-resistant nephrotic syndrome.

作者信息

Yorgin P D, Krasher J, Al-Uzri A Y

机构信息

Department of Pediatrics, Lucile Salter Packard Children's Hospital, Stanford University, CA 94305, USA.

出版信息

Pediatr Nephrol. 2001 Mar;16(3):245-50. doi: 10.1007/s004670000494.

Abstract

Although much of the interest in pulse methylprednisolone therapy (PMT) has centered around its use in children with focal segmental glomerulosclerosis, PMT has also been shown to be effective in the treatment of other proteinuric renal diseases. We hypothesized that a PMT-based treatment protocol, derived from the Tune-Mendoza protocol, would effectively induce a more rapid remission in young children with idiopathic steroid-resistant nephrotic syndrome (SRNS). A retrospective analysis was conducted of 11 consecutive SRNS patients (mean age 3.6 +/- 1.5 years) that received PMT between 1 August 1992 and 1 May 1998. The initial mean urinary protein/urinary creatinine ratio (UP/UC, mg/mg) was 8.3 +/- 9.7 and mean estimated creatinine clearance (CCr) 137.7 +/- 47.0 ml/min per 1.73 m2. An average of 24.8 +/- 10.5 PMT doses were given. The mean duration of PMT therapy until remission was 23.4 +/- 29.9 days (median 12 days). Cyclosporine and cyclophosphamide were used to maintain and extend remissions in 5 and 4 patients, respectively. At the conclusion of the study, the mean UP/UC was 0.12 +/- 0.22 and mean CCr 151.8 +/- 39.8 ml/min per 1.73 m2 (no CCr < or = 100 ml/min per 1.73 m2). Of the 11 patients, 9 attained complete remission. Adverse effects were mild and infrequent. This PMT protocol appears to safely and effectively induce remission in young children with SRNS. A future prospective trial that evaluates the efficacy of PMT in young children with SRNS is warranted.

摘要

尽管对脉冲式甲基强的松龙治疗(PMT)的诸多关注集中在其用于治疗局灶节段性肾小球硬化症患儿,但PMT在治疗其他蛋白尿性肾病方面也已显示出有效性。我们推测,源自图恩 - 门多萨方案的基于PMT的治疗方案,将能有效促使患有特发性类固醇抵抗性肾病综合征(SRNS)的幼儿更快地实现缓解。对1992年8月1日至1998年5月1日期间接受PMT治疗的11例连续性SRNS患者(平均年龄3.6±1.5岁)进行了回顾性分析。初始平均尿蛋白/尿肌酐比值(UP/UC,mg/mg)为8.3±9.7,平均估计肌酐清除率(CCr)为每1.73 m² 137.7±47.0 ml/分钟。平均给予24.8±10.5剂PMT。直至缓解的PMT治疗平均持续时间为23.4±29.9天(中位数为12天)。分别有5例和4例患者使用环孢素和环磷酰胺来维持和延长缓解期。在研究结束时,平均UP/UC为0.12±0.22,平均CCr为每1.73 m² 151.8±39.8 ml/分钟(无CCr≤每1.73 m² 100 ml/分钟)。11例患者中有9例实现了完全缓解。不良反应轻微且不常见。该PMT方案似乎能安全有效地促使SRNS幼儿实现缓解。有必要开展一项未来的前瞻性试验来评估PMT对SRNS幼儿的疗效。

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