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I 型唾液酸贮积症患者的光谱光学相干断层扫描。

Spectral optical coherence tomography in a patient with type I sialidosis.

机构信息

Ophthalmic Clinic Jasne Blonia, Lodz, Poland.

出版信息

Med Sci Monit. 2011 Oct;17(10):CS129-31. doi: 10.12659/msm.881971.

Abstract

BACKGROUND

The aim of our study was to analyze spectral optical coherence tomography (SD-OCT) findings in a patient with clinical signs of sialidosis.

CASE REPORT

Fluorescein angiography and spectral optical coherence tomography was performed in a 37-year-old woman using a SD-OCT device with axial resolution of 6 µm. Enzyme assay followed. The patient was diagnosed with type I sialidosis by enzymatic assay. Besides a normal angiogram, a thickened nerve fiber layer was observed on spectral optical coherence tomography.

CONCLUSIONS

The thickened nerve fiber layer was probably caused by accumulation of metabolic products such as sialylated oligosaccharides and glycopeptides, suggesting that SD- OCT, due to its enhanced resolution, can be a useful tool for diagnosis of rare neurological conditions.

摘要

背景

本研究旨在分析一例具有黏脂贮积症临床体征患者的光谱光学相干断层扫描(SD-OCT)结果。

病例报告

对一名 37 岁女性使用轴向分辨率为 6 µm 的 SD-OCT 设备进行荧光素血管造影和光谱光学相干断层扫描,并进行酶分析。患者通过酶分析诊断为黏脂贮积症 I 型。除了正常的血管造影外,光谱光学相干断层扫描还观察到神经纤维层增厚。

结论

神经纤维层增厚可能是由于代谢产物如唾液酸化寡糖和糖肽的积累所致,这表明 SD-OCT 由于其更高的分辨率,可作为诊断罕见神经疾病的有用工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b5a/3539471/d41cdf03c271/medscimonit-17-10-CS129-g001.jpg

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