Steroid-resistant nephrotic syndrome: the influence of race on cyclophosphamide sensitivity
Steroid-resistant nephrotic syndrome: the influence of race on cyclophosphamide sensitivity
复制标题
DOI：
10.1007/s00467-006-0276-2
复制
发表时间：
2006-12-01
影响因子：
3
通讯作者：
Asharam, Kareshma
中科院分区：
文献类型：
--
作者：
Bhimma, Rajendra;Adhikari, Miriam;Asharam, Kareshma
与其他种族相比，肾病综合征（NS）的类固醇耐药（SR）形式在黑人中的结局较差。在这项研究中，223名儿童SRNS，年龄1-16岁，回顾性分析了1976年至2004年期间。治疗方案包括口服环磷酰胺（2-3 mg/kg）联合泼尼松0.5-1 mg/kg（最大60 mg）（n=90）;泼尼松与甲泼尼龙隔日给药（30 mg/kg，最大1 g）和口服环磷酰胺（n=117）;隔日口服强的松，隔日静脉注射三次甲基强的松龙，每月静脉注射环磷酰胺（500-750 mg/m（-2）剂量（-1次/月，7次/月）（n=10）;或环孢素5 mg/kg（-1）/天（-1），调整至谷水平150-200 mg/ml（n=6）。我们比较了临床和生化特征和结果使用不同形式的治疗。共有183例（82.1%）接受了活检; 84例（45.9%）为印度人，99例（54.1%）为黑人。66例（36.1%）有轻微变化的NS，66例（36.1%）有局灶节段性肾小球硬化（FSGS），15例（8.2%）有增生型NS，36例（19.7%）有其他形式的NS。在84例印度儿童活检中，58例（69.0%）完全缓解，其中40例（72.5%）仅口服环磷酰胺和泼尼松治疗。在99名接受活检的黑人儿童中，20名（20.2%）达到完全缓解;口服环磷酰胺和泼尼松治疗的儿童均未达到完全缓解。在40名未接受活检的印度儿童中，仅接受口服泼尼松和环磷酰胺，32名（80%）达到完全缓解。这项研究表明，印度SRNS儿童对治疗的反应优于黑人儿童（69.0%对20.2%）。由于80%的印度SRNS儿童对口服环磷酰胺和泼尼松的试验有反应，我们建议非黑人儿童在进行肾活检前使用口服环磷酰胺治疗。
Steroid-resistant (SR) forms of nephrotic syndrome (NS) have a poorer outcome in blacks compared to other racial groups. In this study, 223 children with SRNS, aged 1-16 years old, were analysed retrospectively for the period 1976-2004. Treatment schedules included oral cyclophosphamide (2-3 mg/kg) with prednisone 0.5-1 mg/kg (maximum 60 mg) only (n=90); prednisone on alternate days with methylprednisolone (30 mg/kg, maximum 1 g) and oral cyclophosphamide (n=117); oral prednisone on alternate days, three doses of intravenous methylprednisolone on alternate days and monthly doses of intravenous cyclophosphamide (500-750 mg m(-2) dose(-1)x7 doses monthly) (n=10); or cyclosporine 5 mg kg(-1) day(-1) adjusted to a trough level of 150-200 mg/ml (n=6). We compared the clinical and biochemical characteristics and outcome using different forms of therapies. A total of 183 (82.1%) underwent biopsy; 84 (45.9%) were Indian and 99 (54.1%) were black. Sixty-six (36.1%) had minimal change NS, 66 (36.1%) had focal segmental glomerulosclerosis (FSGS), 15 (8.2%) had a proliferative form of NS, and 36 (19.7%) had other forms of NS. Of the 84 Indian children biopsied, 58 (69.0%) were in complete remission, including 29 of 40 (72.5%) treated with oral cyclophosphamide and prednisone only. Of the 99 black children who were biopsied, 20 (20.2%) achieved complete remission; none of those treated with oral cyclophosphamide and prednisone only achieved complete remission. Of the 40 Indian children who were not biopsied who received only oral prednisone and cyclophosphamide, 32 (80%) achieved complete remission. This study shows Indian children with SRNS respond better to treatment than black children (69.0 vs. 20.2%). Since 80% of Indian children with SRNS responded to a trial of oral cyclophosphamide and prednisone, we propose the use of oral cyclophosphamide therapy in non-black children before embarking on renal biopsy.