Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression.
Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression.
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DOI：
10.1007/s00467-022-05762-4
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发表时间：
2023-05
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影响因子：
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目前PodoNet注册分析的目的是评价未接受强化免疫抑制（IIS）治疗的儿童类固醇耐药肾病综合征（SRNS）的结局，重点关注自发缓解的可能性和血管紧张素受体阻滞剂对蛋白尿减少的作用。在PodoNet登记研究中确定了95例未接受任何IIS的儿科患者。对67例发病时患有肾病范围蛋白尿的患者进行竞争风险分析，以探索完全或部分缓解或进展为肾衰竭的累积率，按潜在病因（遗传性与非遗传性SRNS）分层。此外，进行考克斯比例风险分析，以确定预测蛋白尿缓解的因素。31例非遗传性SRNS患者中有18例（58.1%）在没有IIS的情况下实现完全缓解，1年时的累积可能性为46.2%，2年时为57.7%。11名儿童持续缓解，只有两名进展为肾衰竭。在遗传亚组（n = 27）中，蛋白尿的完全消退非常罕见，并且从未持续; 6例（21.7%）儿童在3年时进展为肾衰竭。几乎所有儿童（96.8%）接受了降低蛋白尿的肾素-血管紧张素-醛固酮系统（RAAS）拮抗剂治疗。抗蛋白尿治疗后，31例非遗传性SRNS患儿中有7例（22.6%）获得部分缓解，27例遗传性SRNS患儿中有9例（33.3%）获得部分缓解。我们的研究结果表明，自发完全缓解可以发生在相当一部分的儿童与非遗传性SRNS和温和的临床表型。RAAS阻断可增加所有形式SRNS中蛋白尿部分缓解的可能性。图形摘要的更高分辨率版本可作为补充信息在线版本包含补充材料，可在10.1007/s 00467 -022-05762-4获得。
The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction. Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission. Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (n = 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin–angiotensin–aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS. Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS. A higher resolution version of the Graphical abstract is available as Supplementary information The online version contains supplementary material available at 10.1007/s00467-022-05762-4.
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影响因子：
13.2
作者：
Cattran, DC;Rao, P
通讯作者：
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影响因子：
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作者：
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影响因子：
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作者：
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影响因子：
6.1
作者：
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DOI：
10.2215/cjn.01190210
发表时间：
2010-11-01
影响因子：
9.8
作者：
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通讯作者：
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