Kidney growth progression patterns in autosomal dominant polycystic kidney disease

IF 4.7 3区 医学 Q1 MEDICINE, RESEARCH & EXPERIMENTAL
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引用次数: 0

Abstract

Background

Prognosis for autosomal dominant polycystic kidney disease (ADPKD), the main inherited cause of kidney failure, relies on estimating cystic growth using linear formulas derived from height-adjusted total kidney volume (Ht-TKV). However, nonlinear renal growth patterns may occur in typical ADPKD.

Aims

To determine kidney outcomes of subjects diagnosed with typical ADPKD exhibiting nonlinear, and unpredictable cystic growth during follow-up.

Methods

Retrospective cohort study. We categorized TKV changes in individuals with typical ADPKD according to observed kidney growth trajectories. Ht-TKV was calculated from consecutive CT or MRI using the ellipsoid method. We compared estimated glomerular filtration rate (eGFR) trajectories with linear mixed models.

Results

We included 83 individuals with ADPKD (67% women; age 47 ± 12 years; follow-up 5.2 years [IQR 2.8–9.0]). Three kidney growth patterns were observed: slow progression (24%, <3%/year linear increase), fast progression (39%, ≥3%/year linear increase), and atypical progression (37%, nonlinear growth). Adjusted ht-TKV change in mL/m/year was +1.4 (IQR –4.5 to +10.0), +40.3 (+16.9 to +89.3), and +32.8 (+15.9 to +85.9) for slow, fast, and atypical progressors, respectively (p < 0.001). Atypical progressors exhibited a significantly greater decline in eGFR in mL/min/m²/year (–7.9, 95% CI –6.5, –3.9) compared to slow (–0.5, 95% CI –3.1 to +0.5) and fast progressors (–3.4, 95% CI –7.9, –2.0; between-group p < 0.001). Atypical progressors had a higher proportion of acute complications, including hemorrhages, infections, and urolithiasis (84%), compared to slow (20%) and fast progressors (31%) (p < 0.001).

Conclusion

In typical ADPKD, nonlinear, abrupt, and unpredictable cyst growth occurs frequently, leading to a higher risk of acute complications and kidney function decline.
常染色体显性多囊肾的肾脏发育模式
背景:常染色体显性多囊肾病(ADPKD)是导致肾衰竭的主要遗传性疾病,其预后依赖于根据身高调整肾脏总体积(Ht-TKV)得出的线性公式来估计囊肿的生长情况。然而,在典型的 ADPKD 中可能会出现非线性的肾脏生长模式。目的:确定被诊断为典型 ADPKD 的受试者在随访期间表现出非线性和不可预测的囊性生长的肾脏预后:方法:回顾性队列研究。我们根据观察到的肾脏生长轨迹对典型 ADPKD 患者的 TKV 变化进行了分类。Ht-TKV采用椭圆体法从连续CT或MRI中计算得出。我们用线性混合模型比较了估计的肾小球滤过率(eGFR)轨迹:我们纳入了 83 名 ADPKD 患者(67% 为女性;年龄 47 ± 12 岁;随访 5.2 年 [IQR 2.8-9.0])。观察到三种肾脏生长模式:进展缓慢(24%,结论:在典型的 ADPKD 患者中,非肾脏生长模式的肾脏生长速度较慢:在典型的 ADPKD 中,非线性、突发性和不可预测的囊肿生长经常发生,导致急性并发症和肾功能下降的风险较高。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Archives of Medical Research
Archives of Medical Research 医学-医学:研究与实验
CiteScore
12.50
自引率
0.00%
发文量
84
审稿时长
28 days
期刊介绍: Archives of Medical Research serves as a platform for publishing original peer-reviewed medical research, aiming to bridge gaps created by medical specialization. The journal covers three main categories - biomedical, clinical, and epidemiological contributions, along with review articles and preliminary communications. With an international scope, it presents the study of diseases from diverse perspectives, offering the medical community original investigations ranging from molecular biology to clinical epidemiology in a single publication.
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