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对于患有典型特发性肾病综合征的青少年,是否进行活检?先开始使用类固醇药物。

To biopsy or not to biopsy a teenager with typical idiopathic nephrotic syndrome? Start steroids first.

作者信息

Boyer Olivia, Bernardi Silvia, Preka Evgenia

机构信息

Néphrologie Pédiatrique, Centre de Référence du Syndrome Néphrotique Idiopathique de L'enfant Et L'adulte, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique - Hôpitaux de Paris (APHP), Institut Imagine, INSERM U1163, Université Paris Cité, 149 Rue de Sèvres, 75015, Paris, France.

INSERM U970, PARCC, Paris Translational Research Centre for Organ, Transplantation, Paris, France.

出版信息

Pediatr Nephrol. 2025 Feb;40(2):579-585. doi: 10.1007/s00467-024-06447-w. Epub 2024 Sep 11.

Abstract

It is well known that minimal change disease (MCD) and focal segmental glomerulosclerosis are the most common histopathology findings in children with idiopathic nephrotic syndrome. Moreover, several studies demonstrated that MCD is associated with high steroid-responsiveness and a low incidence of kidney failure, suggesting that routine kidney biopsy is not warranted. Over time, the indications for performing a kidney biopsy have become increasingly stringent, aiming to limit unnecessary invasive procedures in the paediatric population. The most recent guidelines state that a kidney biopsy is not usually necessary at disease onset. Still, it should be performed in case of atypical features suggestive of systemic diseases or glomerulonephritis and in case of steroid-resistance, to assess the different differential diagnoses, regardless of patient age. Moreover, it has been shown that the best prognostic marker in childhood nephrotic syndrome is response to treatment and that kidney histology is not accurate in predicting prognosis. Furthermore, a kidney biopsy is not necessary to predict the relapsing course. Notably, kidney biopsy is an invasive procedure and may lead to significant complications. Finally, novel non-invasive biomarkers have been validated or are in the process of being approved to guide differential diagnoses and thus limit the need for kidney biopsies in patients with typical nephrotic syndrome. In the following sections, we aim to explain why initiating steroid treatment as the initial approach in teenagers with typical nephrotic syndrome is a reasonable strategy. Additionally, we explore how kidney biopsy indications may be alleviated in this population.

摘要

众所周知,微小病变病(MCD)和局灶节段性肾小球硬化是特发性肾病综合征患儿最常见的组织病理学表现。此外,多项研究表明,MCD与高类固醇反应性和低肾衰竭发生率相关,这表明常规肾活检并无必要。随着时间的推移,进行肾活检的指征越来越严格,旨在限制儿科人群中不必要的侵入性操作。最新指南指出,在疾病发作时通常不需要进行肾活检。然而,对于提示全身性疾病或肾小球肾炎的非典型特征以及类固醇抵抗的情况,无论患者年龄如何,都应进行肾活检以评估不同的鉴别诊断。此外,研究表明儿童肾病综合征中最佳的预后标志物是对治疗的反应,而肾脏组织学在预测预后方面并不准确。此外,肾活检对于预测复发过程也没有必要。值得注意的是,肾活检是一种侵入性操作,可能会导致严重并发症。最后,新型非侵入性生物标志物已经得到验证或正在审批过程中,以指导鉴别诊断,从而减少典型肾病综合征患者对肾活检的需求。在以下章节中,我们旨在解释为什么对于患有典型肾病综合征的青少年,将开始使用类固醇治疗作为初始方法是一种合理的策略。此外,我们探讨如何在这一人群中放宽肾活检指征。

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