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维生素 D 缺乏症在肾淀粉样变性患者中普遍存在,并与肾脏预后相关。

Hypovitaminosis D Is Prevalent in Patients With Renal AL Amyloidosis and Associated With Renal Outcome.

机构信息

Division of Hematology, Mayo Clinic, Rochester, MN, United States.

Department of Endocrinology and Kogod Center of Aging, Mayo Clinic College of Medicine, Rochester, MN, United States.

出版信息

Front Endocrinol (Lausanne). 2022 Jun 21;13:891712. doi: 10.3389/fendo.2022.891712. eCollection 2022.

Abstract

INTRODUCTION

Vitamin D deficiency is common, but no data have been reported on vitamin D levels in light chain (AL) amyloidosis.

PATIENTS AND METHODS

In this exploratory study, stored serum samples from 173 patients with newly diagnosed AL amyloidosis were analyzed for vitamin studies which included 25-hydroxyvitamin D [25(OH)D], 1,25-dihydroxyvitamin D [1,25(OH)D] and vitamin D binding protein (DBP). Measurements were made by liquid chromatography-tandem mass spectrometry. Kidney survival and overall survival (OS) were assessed in association to vitamin D status.

RESULTS

Cardiac and kidney involvement occurred in 69% and 63% of patients, respectively. 25(OH)D deficiency (<20 ng/mL) was seen in 56.6% of the patients and was notably found among patients with heavy proteinuria (96%), hypoalbuminemia (84.3%) and morbidly obese patients (68.3%). Heavy proteinuria (>5 gr/24-h) and vitamin D supplementation were independent predictors of 25(OH)D level on nominal multivariate regression analysis. 1,25(0H)D deficiency was noted in 37.6% of patients and was independently associated with low eGFR and hypoalbuminemia. Progression to ESRD occurred in 23.7% of evaluable patients. Patients who progressed to ESRD had lower serum 25(OH)D and 1,25(OH)D levels compared to those who did not progress to ESRD. On a multivariate analysis, severe 25(OH)D deficiency was an independent predictor of progression to ESRD as was renal stage, while 1,25(OH)D deficiency was not.

CONCLUSIONS

Hypovitaminosis D is common in AL amyloidosis, particularly among patients with heavy proteinuria. Severe 25(OH)D deficiency at time of diagnosis predicts progression to ESRD.

摘要

简介

维生素 D 缺乏很常见,但尚无关于轻链(AL)淀粉样变性患者维生素 D 水平的数据报告。

患者和方法

在这项探索性研究中,对 173 例新诊断的 AL 淀粉样变性患者的储存血清样本进行了维生素研究分析,其中包括 25-羟维生素 D [25(OH)D]、1,25-二羟维生素 D [1,25(OH)D]和维生素 D 结合蛋白(DBP)。通过液相色谱-串联质谱法进行测量。评估维生素 D 状态与肾脏生存和总体生存(OS)的关系。

结果

心脏和肾脏受累分别发生在 69%和 63%的患者中。56.6%的患者存在 25(OH)D 缺乏(<20ng/ml),尤其是在大量蛋白尿(96%)、低白蛋白血症(84.3%)和病态肥胖患者(68.3%)中发现。大量蛋白尿(>5g/24h)和维生素 D 补充是名义多变量回归分析中 25(OH)D 水平的独立预测因素。37.6%的患者存在 1,25(0H)D 缺乏,与低 eGFR 和低白蛋白血症独立相关。可评估患者中 23.7%进展为 ESRD。与未进展为 ESRD 的患者相比,进展为 ESRD 的患者血清 25(OH)D 和 1,25(OH)D 水平较低。多变量分析显示,严重 25(OH)D 缺乏是进展为 ESRD 的独立预测因素,与肾脏分期一样,而 1,25(OH)D 缺乏则不是。

结论

AL 淀粉样变性患者中普遍存在维生素 D 缺乏症,尤其是大量蛋白尿患者。诊断时严重的 25(OH)D 缺乏症可预测进展为 ESRD。

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