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Wilson 病患者的光学相干断层扫描。

Optical coherence tomography in patients with Wilson's disease.

机构信息

Department of Neurology, Guangzhou, Guangdong, China.

出版信息

Brain Behav. 2023 Jun;13(6):e3014. doi: 10.1002/brb3.3014. Epub 2023 Apr 16.

Abstract

BACKGROUND

Morphological changes of retina in patients with Wilson's disease (WD) can be found by optical coherence tomography (OCT), and such changes had significant differences between neurological forms (NWD) and hepatic forms (HWD) of WD. The aim of this study was to evaluate the relationship between morphological parameters of retina and brain magnetic resonance imaging (MRI) lesions, course of disease, type of disease, and sexuality in WD.

METHODS

A total of 46 WD patients and 40 health controls (HC) were recruited in this study. A total of 42 WD patients were divided into different groups according to clinical manifestations, course of disease, sexuality, and brain MRI lesions. We employed the Global Assessment Scale to assess neurological severity of WD patients. All WD patients and HC underwent retinal OCT to assess the thickness of inner limiting membrane (ILM) layer to retinal pigment epithelium layer and inner retina layer (ILM to inner plexiform layer, ILM-IPL).

RESULTS

Compared to HWD, NWD had thinner superior parafovea zone (108.07 ± 6.89 vs. 114.40 ± 5.54 μm, p < .01), temporal parafovea zone (97.17 ± 6.65 vs. 103.60 ± 4.53 μm, p < .01), inferior parafovea zone (108.114 ± 7.65 vs. 114.93 ± 5.84 μm, p < .01), and nasal parafovea zone (105.53 ± 8.01 vs. 112.10 ± 5.44 μm, p < .01) in inner retina layer. Course of disease influenced the retina thickness. Male patients had thinner inner retina layer compared to female patients.

CONCLUSION

Our results demonstrated that WD had thinner inner retina layer compared to HC, and NWD had thinner inner retina layer compared to HWD. We speculated the thickness of inner retina layer may be a potential useful biomarker for NWD.

摘要

背景

Wilson 病(WD)患者的视网膜形态变化可通过光学相干断层扫描(OCT)发现,并且这种变化在 WD 的神经型(NWD)和肝型(HWD)之间存在显著差异。本研究旨在评估 WD 患者的视网膜形态参数与脑磁共振成像(MRI)病变、病程、疾病类型和性别之间的关系。

方法

本研究共纳入 46 例 WD 患者和 40 名健康对照者(HC)。根据临床表现、病程、性别和脑 MRI 病变,将 42 例 WD 患者分为不同组。我们采用全球评估量表评估 WD 患者的神经严重程度。所有 WD 患者和 HC 均行视网膜 OCT 检查,以评估内界膜(ILM)层至视网膜色素上皮层和内视网膜层(ILM 至内丛状层,ILM-IPL)的厚度。

结果

与 HWD 相比,NWD 的上旁中心凹区(108.07±6.89 与 114.40±5.54μm,p<.01)、颞旁中心凹区(97.17±6.65 与 103.60±4.53μm,p<.01)、下旁中心凹区(108.114±7.65 与 114.93±5.84μm,p<.01)和鼻旁中心凹区(105.53±8.01 与 112.10±5.44μm,p<.01)内视网膜层厚度较薄。病程影响视网膜厚度。男性患者的内视网膜层厚度较女性患者薄。

结论

我们的研究结果表明,与 HC 相比,WD 的内视网膜层较薄,与 HWD 相比,NWD 的内视网膜层较薄。我们推测内视网膜层的厚度可能是 NWD 的一个潜在有用的生物标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9098/10275519/86a1d71570ba/BRB3-13-e3014-g001.jpg

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