在早期接受治疗的成年人中,神经血管视网膜损伤患有基尿症
Rosa Buonamassa1, Giacomo Boscia1, Marida Gaudiomonte1
1Department of Translational Biomedicine Neuroscience, University of Bari "Aldo Moro", Bari, Italy.
Frontiers in neurology
|July 8, 2024
概括
早期接受治疗的患有基尿症 (ETPKU) 的成年人表现出周周状视网膜神经纤维层厚度降低. 光学连贯断层扫描血管造影显示,ETPKU患者的辐射周毛细血管 perfusion增加,这表明了新的视网膜生物标志物.
科学领域:
- 眼科医生 眼科 眼科
- 代谢障碍 代谢障碍 代谢障碍
- 神经科学是一个神经科学.
背景情况:
- 基尿症 (PKU) 是一种遗传性疾病,需要及早治疗以预防神经损伤.
- 早期治疗PKU对眼睛微血管和视网膜神经纤维层的长期影响尚未完全理解.
- 辐射周周毛细管 (RPC) 和周周毛细管视网膜神经纤维层 (pRNFL) 对于视觉功能至关重要.
研究的目的:
- 为了比较RPC血管参数和pRNFL厚度,在早期治疗的患有PKU (ETPKU) 的成年人和健康的对照人群之间.
- 调查潜在的眼部生物标志物,以监测PKU进展或并发症.
主要方法:
- 一个单心的病例对照研究包括36名参与者的36只眼睛 (18个ETPKU,18个对照).
- 扫描源光学连贯断层扫描血管学 (SS-OCTA) 用于评估RPC血管密度 (VD) 和pRNFL厚度.
- 分析了标准的OCT和OCTA参数.
主要成果:
- 与对照组相比,ETPKU参与者显著减少了pRNFL平均厚度 (p=0.046).
- 小型RPC船舶的平均VD%在ETPKU (52.31%) 和对照 (50.71%) (p=0.049) 中较高.
- 在ETPKU中,与对照组相比 (55.08%) 的ETPKU (58.5%) 显著增加了整体RPC复合体VD% (p<0.001).
结论:
- 结构性OCT和OCTA揭示了视网膜神经纤维的稀薄以及ETPKU中RPCplexus perfusion的增加.
- 在ETPKU中,OCTA可以作为一种非侵入性工具来识别新的视网膜生物标志物.
- 这些发现表明ETPKU患者视网膜的潜在微血管和结构变化.
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