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马凡综合征患儿脉络膜毛细血管血流不足与脉络膜厚度的定量分析。

Quantitative analysis of choriocapillaris flow deficits and choroidal thickness in children with Marfan syndrome.

机构信息

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangzhou, Guangdong, China.

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangzhou, Guangdong, China

出版信息

Br J Ophthalmol. 2024 Jan 29;108(2):274-279. doi: 10.1136/bjo-2022-322535.

Abstract

PURPOSE

To evaluate the characteristics and associated factors of choroidal thickness (ChT) and choriocapillaris flow deficit percentage (CC FD%) in children with Marfan syndrome (MFS).

METHODS

This is a cross-sectional study. The ChT of the subfoveal area and other areas 0.5, 1.5, and 2.5 mm away from the fovea were assessed. The CC FD% of quadrant and circular regions with diameters of 0.5, 1.5 and 2.5 mm were assessed with 3×3 mm macular choriocapillaris images. Best-corrected visual acuity (BCVA) and cardiac function factor such as Z score were measured to analyse the associations with ChT and CC FD%.

RESULTS

51 MFS children and 50 healthy controls were enrolled in this study. Compared with the healthy controls, the ChT in the MFS group was thinner in the subfoveal area, temporal 0.5, 1.5 and 2.5 mm (all p<0.001). The CC FD% was higher in circle 0.5, 1.5 and 2.5 mm (all p<0.001). Multivariate regression analysis showed that CC FD% in the circle 2.5 mm when the Z score ≥2 was associated with BCVA (=9.08 (95% CI 3.96 to 14.20); p=0.005) and Z score (=4.19 (95% CI 1.28 to 12.00); p=0.012).

CONCLUSIONS

Thinner ChT and a higher CC FD% were observed in children with MFS, and an increased CC FD% in circle 2.5 mm was significantly associated with worse BCVA and cardiac function. These findings may help identify future visual impairment and early cardiac events in MFS children.

摘要

目的

评估马凡综合征(MFS)患儿脉络膜厚度(ChT)和脉络膜毛细血管血流缺损百分比(CC FD%)的特征及其相关因素。

方法

这是一项横断面研究。评估了黄斑中心凹下区及距黄斑中心凹 0.5、1.5 和 2.5mm 处的脉络膜厚度。采用 3×3mm 黄斑脉络膜毛细血管图像评估直径为 0.5、1.5 和 2.5mm 的象限和圆形区域的 CC FD%。测量最佳矫正视力(BCVA)和心脏功能因子如 Z 评分,以分析与 ChT 和 CC FD%的相关性。

结果

本研究纳入了 51 名 MFS 患儿和 50 名健康对照者。与健康对照组相比,MFS 组的黄斑中心凹下区、颞侧 0.5、1.5 和 2.5mm 的脉络膜厚度较薄(均 p<0.001)。0.5、1.5 和 2.5mm 圆形区域的 CC FD%较高(均 p<0.001)。多变量回归分析显示,Z 评分≥2 时 2.5mm 圆形区域的 CC FD%与 BCVA 相关(=9.08(95%CI 3.96 至 14.20);p=0.005)和 Z 评分(=4.19(95%CI 1.28 至 12.00);p=0.012)。

结论

MFS 患儿的脉络膜厚度较薄,脉络膜毛细血管血流缺损百分比较高,2.5mm 圆形区域的脉络膜毛细血管血流缺损百分比与 BCVA 和心脏功能显著相关。这些发现可能有助于识别 MFS 患儿未来的视力损害和早期心脏事件。

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