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常染色体显性多囊肾病患者肾衰竭时的高级影像学生物标志物评估:一项试点研究。

Evaluation of advanced imaging biomarkers at kidney failure in patients with ADPKD: a pilot study.

作者信息

Wigerinck Stijn, Gregory Adriana V, Smith Byron H, Iliuta Ioan-Andrei, Hanna Christian, Chedid Maroun, Kaidbay Hasan-Daniel N, Senum Sarah R, Shukoor Shebaz, Harris Peter C, Torres Vicente E, Kline Timothy L, Chebib Fouad T

机构信息

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.

Faculty of Medicine, Catholic University of Leuven, Leuven, Belgium.

出版信息

Clin Kidney J. 2023 May 23;16(10):1691-1700. doi: 10.1093/ckj/sfad114. eCollection 2023 Oct.

Abstract

BACKGROUND

Autosomal dominant polycystic kidney disease (ADPKD) presents with variable disease severity and progression. Advanced imaging biomarkers may provide insights into cystic and non-cystic processes leading to kidney failure in different age groups.

METHODS

This pilot study included 39 ADPKD patients with kidney failure, stratified into three age groups (<46, 46-56, >56 years old). Advanced imaging biomarkers were assessed using an automated instance cyst segmentation tool. The biomarkers were compared with an age- and sex-matched ADPKD cohort in early chronic kidney disease (CKD).

RESULTS

Ht-total parenchymal volume correlated negatively with age at kidney failure. The median Ht-total parenchymal volume was significantly lower in patients older than 56 years. Cystic burden was significantly higher at time of kidney failure, especially in patients who reached it before age 46 years. The cyst index at kidney failure was comparable across age groups and Mayo Imaging Classes. Advanced imaging biomarkers showed higher correlation with Ht-total kidney volume in early CKD than at kidney failure. Cyst index and parenchymal index were relatively stable over 5 years prior to kidney failure, whereas Ht-total cyst volume and cyst parenchymal surface area increased significantly.

CONCLUSION

Age-related differences in advanced imaging biomarkers suggest variable pathophysiological mechanisms in ADPKD patients with kidney failure. Further studies are needed to validate the utility of these biomarkers in predicting disease progression and guiding treatment strategies.

摘要

背景

常染色体显性多囊肾病(ADPKD)的疾病严重程度和进展各不相同。先进的影像生物标志物可能有助于深入了解导致不同年龄组肾衰竭的囊性和非囊性病变过程。

方法

这项前瞻性研究纳入了39例患有肾衰竭的ADPKD患者,分为三个年龄组(<46岁、46 - 56岁、>56岁)。使用自动实例囊肿分割工具评估先进的影像生物标志物。将这些生物标志物与年龄和性别匹配的早期慢性肾脏病(CKD)ADPKD队列进行比较。

结果

实质总体积与肾衰竭时的年龄呈负相关。56岁以上患者的实质总体积中位数显著更低。肾衰竭时的囊肿负荷显著更高,尤其是在46岁之前发生肾衰竭的患者中。各年龄组和梅奥影像分级的肾衰竭时囊肿指数相当。与肾衰竭时相比,先进的影像生物标志物在早期CKD时与实质总体积的相关性更高。肾衰竭前5年囊肿指数和实质指数相对稳定,而囊肿总体积和囊肿实质表面积显著增加。

结论

先进影像生物标志物的年龄相关差异表明,患有肾衰竭的ADPKD患者存在不同的病理生理机制。需要进一步研究以验证这些生物标志物在预测疾病进展和指导治疗策略方面的效用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ceac/10539251/a23c83ec6932/sfad114fig1g.jpg

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