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观察Leber遗传性视神经病变自然病程及基因治疗后视乳头周围脉络膜血管情况

Observation of Peripapillary Choroidal Vascularity in Natural Disease Course and After Gene Therapy for Leber's Hereditary Optic Neuropathy.

作者信息

Jiang Jingwen, Sun Gongpeng, Miao Qingmei, Li Bin, Wang Dan, Yuan Jiajia, Chen Changzheng

机构信息

Eye Center, Renmin Hospital of Wuhan University, Wuhan, China.

Department of Ophthalmology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

出版信息

Front Med (Lausanne). 2021 Dec 7;8:770069. doi: 10.3389/fmed.2021.770069. eCollection 2021.

DOI:10.3389/fmed.2021.770069
PMID:34950684
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8691380/
Abstract

To compare peripapillary choroidal vascularity among Leber's Hereditary Optic Neuropathy (LHON) patients at different stages of natural course and healthy controls using optical coherence tomography (OCT), and to evaluate peripapillary choroidal vascularity changes in LHON patients before and after gene therapy. 57 LHON patients and 15 healthy controls were enrolled in this prospective clinical study. LHON patients were divided into three duration groups based on stage of disease progression. Both patients and healthy controls underwent OCT scans focused on the optic disc at baseline with Heidelberg Spectralis, and patients underwent OCT at 1, 3, and 6 months after gene therapy. OCT images were converted and binarized using ImageJ software. Choroidal thickness (CT), total choroidal area (TCA), and choroidal vascularity index (CVI) in each quadrant of OCT images were measured to evaluate peripapillary choroidal vascularity. At baseline, the average CT was not significantly different between LHON patients at different stages and between healthy controls ( = 0.468). Although average TCA and average CVI were slightly higher in LHON patients at different stages than in healthy controls, the difference was not statistically significant ( = 0.282 and 0.812, respectively). After gene therapy, The average TCA at 1 month after gene therapy was significantly higher than that before gene therapy ( = 0.003), while no significant differences were found in the average CT or average CVI in LHON patients before and 1,3 and 6 months after gene therapy using pairwise comparisons (all > 0.05). No significant difference was found in choroidal vascularity of LHON patients at different stages and healthy controls. Choroidal vascularity seems to stay stable after gene therapy.

摘要

使用光学相干断层扫描(OCT)比较Leber遗传性视神经病变(LHON)患者自然病程不同阶段与健康对照者的视乳头周围脉络膜血管情况,并评估LHON患者基因治疗前后视乳头周围脉络膜血管的变化。57例LHON患者和15名健康对照者纳入了这项前瞻性临床研究。LHON患者根据疾病进展阶段分为三个病程组。患者和健康对照者在基线时均使用海德堡Spectralis对视盘进行OCT扫描,患者在基因治疗后1、3和6个月进行OCT检查。使用ImageJ软件对OCT图像进行转换和二值化处理。测量OCT图像各象限的脉络膜厚度(CT)、脉络膜总面积(TCA)和脉络膜血管指数(CVI),以评估视乳头周围脉络膜血管情况。基线时,不同阶段的LHON患者与健康对照者之间的平均CT无显著差异(P = 0.468)。虽然不同阶段的LHON患者平均TCA和平均CVI略高于健康对照者,但差异无统计学意义(分别为P = 0.282和0.812)。基因治疗后,基因治疗后1个月的平均TCA显著高于基因治疗前(P = 0.003),而使用两两比较时,LHON患者基因治疗前与基因治疗后1、3和6个月的平均CT或平均CVI均无显著差异(均P > 0.05)。不同阶段的LHON患者与健康对照者的脉络膜血管情况无显著差异。基因治疗后脉络膜血管情况似乎保持稳定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/8c02e2cff216/fmed-08-770069-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/3932e511a9ea/fmed-08-770069-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/52de0c976d35/fmed-08-770069-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/8c02e2cff216/fmed-08-770069-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/3932e511a9ea/fmed-08-770069-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/52de0c976d35/fmed-08-770069-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5174/8691380/8c02e2cff216/fmed-08-770069-g0003.jpg

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Exploring choroidal angioarchitecture in health and disease using choroidal vascularity index.
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