常染色体显性多囊肾病预测工具和风险预测的综合应用。

IF 8.5 1区医学 Q1 UROLOGY & NEPHROLOGY

Clinical Journal of the American Society of Nephrology Pub Date : 2025-03-01 Epub Date: 2024-12-20 DOI:10.2215/CJN.0000000625

Constantin A Wolff, Valeria Aiello, Elhussein A E Elhassan, Carlotta Cristalli, Sarah Lerario, Alexandro Paccapelo, Francesca Ciurli, Francesca Montanari, Amalia Conti, Katherine Benson, Marco Seri, Carolin B Brigl, Julia S Münster, Nicola Sciascia, Sebastian Kursch, Jonathan de Fallois, Gaetano La Manna, Kai-Uwe Eckardt, Nina Rank, Bernt Popp, Ria Schönauer, Peter J Conlon, Irene Capelli, Jan Halbritter

引用次数: 0

摘要

背景：常染色体显性多囊肾病（ADPKD）是肾衰竭最常见的遗传原因。在观察到或预测到快速进展时，需要进行特殊治疗。对于后者，风险分层工具已经根据肾脏总容量或基因分型以及临床变量独立开发。本研究旨在通过结合影像学和临床遗传学评分来提高风险预测。方法：我们对468例确诊为ADPKD的患者进行了回顾性多中心队列研究。对临床、影像学和遗传数据进行分析以预测风险。我们将疾病快速进展定义为两年内肾小球滤过率（eGFR）斜率≥3ml /min/1.73m2/年，Mayo影像分类（MIC） 1D-1E，或多囊肾病预测肾结局（PROPKD）评分≥7分。使用MIC、PROPKD和Rare Exome Variant Ensemble Learner （REVEL）评分，设计了几个组合模型，以开发具有改进风险分层的新分类。主要终点为晚期慢性肾病（aCKD） G4-G5期的发展、eGFR的纵向变化以及高血压或泌尿系统事件等临床变量。统计学上采用logistic回归、生存、受试者工作特征（ROC）分析、线性混合模型和Cox比例风险模型。结论：两种风险评分的联合使用与更高的识别快速进展的能力相关，并导致更好的分层，特别是在中等风险患者中。

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来源期刊

Clinical Journal of the American Society of Nephrology 医学-泌尿学与肾脏学

CiteScore

12.20

自引率

3.10%

发文量

514

审稿时长

3-6 weeks

期刊介绍： The Clinical Journal of the American Society of Nephrology strives to establish itself as the foremost authority in communicating and influencing advances in clinical nephrology by (1) swiftly and effectively disseminating pivotal developments in clinical and translational research in nephrology, encompassing innovations in research methods and care delivery; (2) providing context for these advances in relation to future research directions and patient care; and (3) becoming a key voice on issues with potential implications for the clinical practice of nephrology, particularly within the United States. Original manuscript topics cover a range of areas, including Acid/Base and Electrolyte Disorders, Acute Kidney Injury and ICU Nephrology, Chronic Kidney Disease, Clinical Nephrology, Cystic Kidney Disease, Diabetes and the Kidney, Genetics, Geriatric and Palliative Nephrology, Glomerular and Tubulointerstitial Diseases, Hypertension, Maintenance Dialysis, Mineral Metabolism, Nephrolithiasis, and Transplantation.