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有神经学表现的肝豆状核变性患者血浆中胶质纤维酸性蛋白浓度较高。

Higher Concentration of Plasma Glial Fibrillary Acidic Protein in Wilson Disease Patients with Neurological Manifestations.

作者信息

Lin Jie, Zheng Yexiang, Liu Ying, Lin Yi, Wang Qiqi, Lin Xiao-Hong, Zhu Wenli, Lin Wei-Hong, Wang Ning, Chen Wan-Jin, Fu Ying

机构信息

Department of Neurology and Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Medical University, Fuzhou, China.

Department of Radiology of First Affiliated Hospital, Fujian Medical University, Fuzhou, China.

出版信息

Mov Disord. 2021 Jun;36(6):1446-1450. doi: 10.1002/mds.28509. Epub 2021 Jan 27.

DOI:10.1002/mds.28509
PMID:33502774
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8248415/
Abstract

BACKGROUND

Wilson disease is a rare, disabling, neurological genetic disease. Biomarkers of brain damage are less well developed.

OBJECTIVE

The aim of this study was to evaluate the utility of plasma glial fibrillary acidic protein as a biomarker for neurological involvement in patients with Wilson disease.

METHODS

This prospective cross-observational study compared plasma glial fibrillary acidic protein concentration among different subtypes of patients with Wilson disease and healthy control subjects. Plasma glial fibrillary acidic protein levels were measured in 94 patients and 25 healthy control subjects. Patients were divided into two subtypes: patients with neurological manifestations (n = 74) or hepatic manifestations (n = 20).

RESULTS

Median levels of plasma glial fibrillary acidic protein were significantly elevated in patients with neurological manifestations (143.87 pg/mL) compared with those with hepatic manifestations (107.50 pg/mL) and healthy control subjects (86.85 pg/mL). Receiver operating characteristic curve revealed that a plasma glial fibrillary acidic protein cutoff value of 128.8 pg/mL provides sufficient sensitivity (80.0%) and specificity (63.5%) to differentiate patients with neurological manifestations from those with hepatic manifestations.

CONCLUSIONS

Plasma glial fibrillary acidic protein may serve as a biomarker for distinguishing different subtypes of Wilson disease. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

摘要

背景

威尔逊病是一种罕见的、致残性神经遗传病。脑损伤生物标志物的研究尚不完善。

目的

本研究旨在评估血浆胶质纤维酸性蛋白作为威尔逊病患者神经受累生物标志物的效用。

方法

这项前瞻性交叉观察研究比较了威尔逊病不同亚型患者与健康对照者的血浆胶质纤维酸性蛋白浓度。对94例患者和25例健康对照者测量了血浆胶质纤维酸性蛋白水平。患者分为两个亚型:有神经表现的患者(n = 74)和有肝脏表现的患者(n = 20)。

结果

有神经表现的患者血浆胶质纤维酸性蛋白的中位数水平(143.87 pg/mL)显著高于有肝脏表现的患者(107.50 pg/mL)和健康对照者(86.85 pg/mL)。受试者工作特征曲线显示,血浆胶质纤维酸性蛋白临界值为128.8 pg/mL时,具有足够的敏感性(80.0%)和特异性(63.5%)来区分有神经表现的患者和有肝脏表现的患者。

结论

血浆胶质纤维酸性蛋白可作为区分威尔逊病不同亚型的生物标志物。© 2021作者。由Wiley Periodicals LLC代表国际帕金森和运动障碍协会出版的《运动障碍》。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fe0/8248415/7d51fceade99/MDS-36-1446-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fe0/8248415/7d51fceade99/MDS-36-1446-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fe0/8248415/7d51fceade99/MDS-36-1446-g001.jpg

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