儿童视网膜变性：暗适应视觉阈值与小动脉直径
Retinal degeneration in children: dark adapted visual threshold and arteriolar diameter.
作者信息
Hansen Ronald M, Eklund Susan E, Benador Ilan Y, Mocko Julie A, Akula James D, Liu Yao, Martinez-Perez M Elena, Fulton Anne B
机构信息
Department of Ophthalmology, Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
出版信息
Vision Res. 2008 Feb;48(3):325-31. doi: 10.1016/j.visres.2007.07.009. Epub 2007 Aug 31.
To assess the condition of the retina in children with retinal degeneration due to Bardet-Biedl syndrome (BBS, n=41), Leber congenital amaurosis (LCA, n=31), or Usher syndrome (USH, n=13), the dark adapted visual threshold (DAT) and arteriolar diameters were measured. Compared to controls, the initial DATs of nearly all (83/85) were significantly elevated, and in 26/62 with serial DATs, significant progressive elevation occurred. Arteriolar diameters were significantly attenuated and narrowed with age in BBS and USH, but not LCA. Higher DATs were associated with narrower arterioles. Such non-invasive procedures can document the natural history of these retinal diseases and have the potential to assess response to future treatment.
为评估患有因巴德-比德尔综合征（BBS，n = 41）、莱伯先天性黑矇（LCA，n = 31）或Usher综合征（USH，n = 13）导致视网膜变性的儿童的视网膜状况，测量了暗适应视觉阈值（DAT）和小动脉直径。与对照组相比，几乎所有（83/85）患者的初始DAT均显著升高，并且在62例有连续DAT测量值的患者中，有26例出现了显著的进行性升高。在BBS和USH患者中，小动脉直径随年龄显著变细和变窄，但在LCA患者中并非如此。较高的DAT与较窄的小动脉相关。此类非侵入性检查可记录这些视网膜疾病的自然病程，并有可能评估对未来治疗的反应。
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