在IgA肾病儿童队列中,真实肾脏预后与诊断时和活检后预测预后的比较

IF 1.3 Q3 UROLOGY & NEPHROLOGY
Mafalda Félix Cabral, Inês Martins, Miguel Pereira, Gonçalo Vale, Madalena Almeida Borges, Maria Soto-Maior Costa, Fernando Caeiro, Mário Góis, Helena Sousa, Telma Francisco, Gisela Neto, Margarida Abranches, Rute Baeta Baptista
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引用次数: 0

摘要

简介:IgA肾病(IgAN)是原发性肾小球肾炎最常见的形式,具有不同的临床病程。本研究旨在评估患有IgAN的儿童的真实肾脏预后,并将其与国际儿童IgAN预测工具(IIgAN-PT)的预测结果进行比较。方法:对2010 - 2022年诊断为IgAN的儿科患者进行单中心纵向回顾性研究。分析了临床、实验室和组织学参数的数据。计算每位患者活检时和活检后一年的IIgAN-PT评分。主要终点为eGFR降低≥30%或进展为终末期肾病(ESKD)的复合终点。结果:在23例患者中(57%为男性,活检时中位年龄13.8岁),MEST-C评分显示M1占87%,E1占22%,S1占39%,T1/2占13%,C1占26%。在中位3.1年的随访期间,26%达到了主要结局,而基于IIgAN-PT的中位预测风险为6.5%。此外,57%的患者eGFR下降(年平均下降5.6 mL/min/1.73 m2)。在活检后1年(n = 13)应用更新的IIgAN-PT,中位预测风险为1.79%,23%达到主要结局。结论:观察到的eGFR下降或进展为ESKD高于预期,突出了早期诊断、监测和治疗的必要性。像我们这样的小规模研究强调了早期干预的重要性,并可能为设计更大规模的研究提供信息,以提高IIgAN-PT工具在不同临床环境中的预测能力。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Comparison of real-world kidney outcomes with predicted outcomes at the time of diagnosis and post-biopsy in a cohort of children with IgA nephropathy.

Introduction: IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis, with a variable clinical course. This study aimed to evaluate real-world kidney outcomes in children with IgAN and compare these with predictions from the International IgAN Prediction Tool for children (IIgAN-PT).

Methods: A single-center, longitudinal retrospective study was conducted on pediatric patients diagnosed with IgAN from 2010 to 2022. Data on clinical, laboratory, and histological parameters were analyzed. The IIgAN-PT score was calculated for each patient at biopsy and one year after biopsy. The primary outcome was a composite endpoint of ≥30% eGFR decrease or progression to end stage kidney disease (ESKD).

Results: Among 23 patients (57% male, median age at biopsy 13.8 years), MEST-C scores showed M1 in 87%, E1 in 22%, S1 in 39%, T1/2 in 13%, and C1 in 26%. During a median 3.1-year follow-up, 26% reached the primary outcome, while the median predicted risk based on IIgAN-PT was 6.5%. Additionally, 57% experienced eGFR decline (annual median decline of 5.6 mL/min/1.73 m2). Application of the updated IIgAN-PT one-year post-biopsy (n = 13) resulted in a median predicted risk of 1.79%, while 23% met the primary outcome.

Conclusion: The observed eGFR decline or progression to ESKD was higher than predicted, highlighting the need for early diagnosis, monitoring, and treatment. Small-scale studies like ours underscore the importance of early intervention and may inform the design of larger studies to improve the predictive ability of the IIgAN-PT tool in diverse clinical settings.

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来源期刊
CiteScore
2.20
自引率
16.70%
发文量
208
审稿时长
16 weeks
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