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儿童激素依赖型肾病综合征的预测风险因素

Predictive risk factors of steroid dependent nephrotic syndrome in children.

作者信息

Abdel-Hafez Maher Ahmed, Abou-El-Hana Nagy Mohamed, Erfan Adel Ali, El-Gamasy Mohamed, Abdel-Nabi Hend

机构信息

Pediatric Department, Faculty of Medicine, Tanta University, Egypt.

出版信息

J Nephropathol. 2017 Jul;6(3):180-186. doi: 10.15171/jnp.2017.31. Epub 2017 Feb 2.

DOI:10.15171/jnp.2017.31
PMID:28975099
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5607981/
Abstract

BACKGROUND

Development of steroid dependency is one of the difficult problems in the management of children with idiopathic nephrotic syndrome, leading to increased morbidity, complications and cost of treatment. Thus, predicting early in the disease course will be useful in counseling parents and may improve treatment strategy.

OBJECTIVES

To determine the clinical characteristics that can predict the development of steroid dependency early in the initial episodes of steroid sensitive nephrotic syndrome (SSNS).

PATIENTS AND METHODS

The study included 52 children with SSNS. Their ages ranged from 3 to 16 years. Patients were divided into two groups. Group A consisted of 24 patients with steroid dependency or frequent relapses nephrotic syndrome and group B consisted of 28 patients with complete remission or recurrent nephrotic syndrome. Data obtained retrospectively from patients' files.

RESULTS

Children who require a cumulative steroid dose equal or more than 140 mg/kg to maintain remission during the first 6 months of the disease are at high risk to require steroid sparing agents (SSA) for disease control, and who did not achieve remission by day 20 of the initial prednisone course became steroid dependent with 96% specificity but with low sensitivity (50%). All steroid dependent children in this study showed relapses associated significantly with upper respiratory tract infections.

CONCLUSIONS

Cumulative steroid dose in the first 6 months of treatment and the need of more than 20 days to achieve initial remission can predict steroid dependency in children with nephrotic syndrome.

摘要

背景

激素依赖的发生是特发性肾病综合征患儿治疗中的难题之一,会导致发病率、并发症及治疗费用增加。因此,在疾病早期进行预测对向家长提供咨询有帮助,且可能改善治疗策略。

目的

确定可在激素敏感型肾病综合征(SSNS)初始发作早期预测激素依赖发生的临床特征。

患者与方法

本研究纳入52例SSNS患儿。年龄范围为3至16岁。患者分为两组。A组由24例激素依赖或频繁复发的肾病综合征患者组成,B组由28例完全缓解或复发的肾病综合征患者组成。数据从患者病历中回顾性获取。

结果

在疾病最初6个月期间需要累积激素剂量等于或超过140mg/kg以维持缓解的患儿,有很高风险需要使用激素替代药物(SSA)来控制疾病,且在初始泼尼松疗程第20天时未达到缓解的患儿会成为激素依赖者,其特异性为96%,但敏感性较低(50%)。本研究中所有激素依赖患儿的复发均与上呼吸道感染显著相关。

结论

治疗前6个月的累积激素剂量以及达到初始缓解需要超过20天的时间,可预测肾病综合征患儿的激素依赖情况。

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Early age at debut is a predictor of steroid-dependent and frequent relapsing nephrotic syndrome.发病年龄早是激素依赖和频繁复发肾病综合征的预测因子。
Pediatr Nephrol. 2010 Jul;25(7):1299-304. doi: 10.1007/s00467-010-1537-7. Epub 2010 May 6.
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Time for initial response to steroids is a major prognostic factor in idiopathic nephrotic syndrome.初始激素治疗反应时间是特发性肾病综合征的一个主要预后因素。
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Long-term outcome of children with steroid-resistant nephrotic syndrome treated with tacrolimus.
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Front Pediatr. 2023 Oct 6;11:1248733. doi: 10.3389/fped.2023.1248733. eCollection 2023.
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Clinical effect of different prednisone regimens in the treatment of children with primary nephrotic syndrome and risk factors for recurrence.不同泼尼松治疗方案对儿童原发性肾病综合征的临床疗效及复发的影响因素分析。
Zhongguo Dang Dai Er Ke Za Zhi. 2022 Aug 15;24(8):853-857. doi: 10.7499/j.issn.1008-8830.2203029.
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Prediction of steroid resistance and steroid dependence in nephrotic syndrome children.儿童肾病综合征中类固醇抵抗和类固醇依赖的预测
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他克莫司治疗类固醇耐药性肾病综合征患儿的长期预后。
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Long-term outcome of children with steroid-sensitive idiopathic nephrotic syndrome.激素敏感型特发性肾病综合征患儿的长期预后
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A prospective study on the use of mycophenolate mofetil in children with cyclosporine-dependent nephrotic syndrome.一项关于霉酚酸酯用于依赖环孢素的儿童肾病综合征的前瞻性研究。
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