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利用体内共聚焦显微镜成像观察肝豆状核变性的凯-弗环

Imaging Kayser-Fleischer Ring in Wilson Disease Using In Vivo Confocal Microscopy.

作者信息

Zhao Tantai, Fang Zhixue, Tian Jiao, Liu Jia, Xiao Yangyan, Li Huiling, Chen Baihua

机构信息

Department of Ophthalmology, The Second Xiangya Hospital, Central South University, Changsha, China.

Hunan Clinical Research Center of Ophthalmic Disease, Changsha, China.

出版信息

Cornea. 2019 Mar;38(3):332-337. doi: 10.1097/ICO.0000000000001844.

DOI:10.1097/ICO.0000000000001844
PMID:30601285
Abstract

PURPOSE

This study analyzes images of Kayser-Fleischer (K-F) rings in patients with Wilson disease (WD) using in vivo confocal microscopy (IVCM) and explores whether IVCM can be a useful clinical tool in facilitating the diagnosis and characterization of K-F rings.

METHODS

One hundred four eyes of 52 patients with WD and K-F rings (K-F group) and 52 normal eyes of 52 age- and gender-matched control subjects (control group) were enrolled in the study. Both K-F and control groups consisted of 29 male patients and 23 female patients. IVCM imaging was performed, and images of the peripheral Descemet membrane were analyzed.

RESULTS

All patients in K-F group showed abnormal patterns in the peripheral Descemet membrane from IVCM images. These abnormalities can be generally divided into 3 types: patchy, stripy, and spotty patterns. Each patient may have a combination of these patterns, with patchy pattern being most prevalent (100%), whereas stripy and spotty patterns are present in 30% to 40% of the K-F rings. Notably, these patterns are not correlated with other systematic symptoms of WD.

CONCLUSIONS

IVCM images can be used as an objective clinical diagnostic tool to facilitate the identification of K-F rings and the diagnosis of WD.

摘要

目的

本研究使用活体共聚焦显微镜(IVCM)分析威尔逊病(WD)患者的凯泽-弗莱舍尔(K-F)环图像,并探讨IVCM是否可作为有助于K-F环诊断和特征描述的有用临床工具。

方法

本研究纳入了52例患有WD且有K-F环的患者的104只眼(K-F组)以及52例年龄和性别匹配的对照受试者的52只正常眼(对照组)。K-F组和对照组均由29例男性患者和23例女性患者组成。进行了IVCM成像,并分析了周边Descemet膜的图像。

结果

K-F组所有患者的IVCM图像显示周边Descemet膜有异常模式。这些异常通常可分为3种类型:斑片状、条纹状和斑点状模式。每位患者可能有这些模式的组合,其中斑片状模式最为普遍(100%),而条纹状和斑点状模式在30%至40%的K-F环中出现。值得注意的是,这些模式与WD的其他系统症状无关。

结论

IVCM图像可作为一种客观的临床诊断工具,有助于K-F环的识别和WD的诊断。

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引用本文的文献

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Comparative evaluation of anterior segment optical coherence tomography and in vivo confocal microscopy for Kayser-Fleischer rings assessment in Wilson disease.眼前节光学相干断层扫描与体内共聚焦显微镜在威尔逊病凯-弗环评估中的比较评价
BMC Ophthalmol. 2025 Apr 15;25(1):209. doi: 10.1186/s12886-025-04032-9.
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Delayed Diagnosis of Wilson's Disease Report From 179 Newly Diagnosed Cases in China.中国179例新诊断威尔逊病患者的延迟诊断报告
Front Neurol. 2022 Jul 5;13:884840. doi: 10.3389/fneur.2022.884840. eCollection 2022.
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Eye Involvement in Wilson's Disease: A Review of the Literature.
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J Clin Med. 2022 Apr 30;11(9):2528. doi: 10.3390/jcm11092528.
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Investigation of corneal topographic and densitometric properties of Wilson's disease patients with or without a Kayser-Fleischer ring.对患有或未患有凯-费环的威尔逊氏病患者的角膜地形图和密度测量特性进行研究。
Eye Vis (Lond). 2021 Mar 11;8(1):8. doi: 10.1186/s40662-021-00231-9.
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Clinical manifestations of Wilson disease in organs other than the liver and brain.肝和脑以外器官的威尔逊病临床表现。
Ann Transl Med. 2019 Apr;7(Suppl 2):S62. doi: 10.21037/atm.2019.03.30.