Fulton A B, Hansen R M, Glynn R J
Department of Ophthalmology, Children's Hospital, Boston, Mass.
Arch Ophthalmol. 1993 Nov;111(11):1500-6. doi: 10.1001/archopht.1993.01090110066026.
To determine the course of visual functions in patients with Bardet-Biedl syndrome.
The 21 patients with Bardet-Biedl syndrome seen at Children's Hospital, Boston, Mass, had optotype and grating acuities and dark-adapted thresholds measured over time. Their ages at first visit ranged from 2 weeks to 23 years (median age, 6 years). The courses of the visual functions were analyzed with a random-effects model.
Substantial declines in visual functions were found. On average, grating and optotype acuities declined 0.09 log units (roughly 1 line) per year, and thresholds increased about 0.19 log units per year. The rates at which these visual functions were lost and the predicted level of the visual functions at ages 11 to 12 years (the mean ages of measurement) varied among individuals.
The visual prognosis for children with Bardet-Biedl syndrome is poor. The course of both central and peripheral visual functions is variable.
确定巴德-比德尔综合征患者的视觉功能变化过程。
对在马萨诸塞州波士顿儿童医院就诊的21例巴德-比德尔综合征患者，随着时间推移测量其视标和光栅视力以及暗适应阈值。他们首次就诊时的年龄从2周龄至23岁不等（中位年龄为6岁）。采用随机效应模型分析视觉功能的变化过程。
发现视觉功能有显著下降。平均而言，光栅和视标视力每年下降0.09对数单位（约1行），阈值每年升高约0.19对数单位。这些视觉功能丧失的速率以及在11至12岁（测量的平均年龄）时视觉功能的预测水平在个体之间存在差异。
巴德-比德尔综合征患儿的视觉预后较差。中枢和周边视觉功能的变化过程各不相同。
